Background: Congenital heart disease (CHD) accounts for approximately 40% of deaths in United States (US) children with birth defects. Previous US data from 1999-2006 demonstrated an overall decrease in CHD mortality. Our study aimed to assess current trends in US mortality related to CHD from infancy to adulthood over the last 19 years and determine differences by sex and race/ethnicity. Methods: We conducted an analysis of death certificates from 1999-2017 to calculate annual CHD mortality by age at death, race/ethnicity, and sex. Population estimates used as denominators in mortality rate calculations for infants were based on National Center for Health Statistics live birth data. Mortality rates in individuals ≥1 year of age utilized US Census Bureau bridged-race estimates as denominators for population estimates. We characterized temporal trends in all-cause mortality, mortality resulting directly due to and related to CHD by age, race/ethnicity, and sex using joinpoint regression. Results: There were 47.7 million deaths with 1 in 814 deaths due to CHD (n=58,599). While all-cause mortality decreased 16.4% across all ages, mortality resulting from CHD declined 39.4% overall. The mean annual decrease in CHD mortality was 2.6%, with the largest decrease for those age >65 years. The age-adjusted mortality rate decreased from 1.37 to 0.83 per 100,000. Males had higher mortality due to CHD than females throughout the study, although both sexes declined at a similar rate (~40% overall), with a 3-4% annual decrease between 1999 and 2009, followed by a slower annual decrease of 1.4% through 2017. Mortality resulting from CHD significantly declined among all races/ethnicities studied, although disparities in mortality persisted for non-Hispanic blacks versus non-Hispanic whites (mean annual decrease 2.3% versus 2.6%, respectively; age-adjusted mortality rate 1.67 to 1.05 versus 1.35 to 0.80 per 100,000, respectively). Conclusions: While overall US mortality due to CHD has decreased over the last 19 years, disparities in mortality persist for males compared to females and for non-Hispanic blacks compared to non-Hispanic whites. Determining factors that contribute to these disparities such as access to quality care, timely diagnosis, and maintenance of insurance will be important moving into the next decade.

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Background: Anomalous aortic origin of a coronary artery (CA) is the second leading cause of sudden cardiac death in young athletes. Management is controversial and longitudinal follow-up data are sparse. We aim to evaluate outcomes in a prospective study of anomalous aortic origin of CA patients following a standardized algorithm. Methods: Patients with anomalous aortic origin of a CA were followed prospectively from December 2012 to April 2017. All patients were evaluated following a standardized algorithm, and data were reviewed by a dedicated multidisciplinary team. Assessment of myocardial perfusion was performed using stress imaging. High-risk patients (high-risk anatomy—anomalous left CA from the opposite sinus, presence of intramurality, abnormal ostium—and symptoms or evidence of myocardial ischemia) were offered surgery or exercise restriction (if deemed high risk for surgical intervention). Univariate and multivariable analyses were used to determine predictors of high risk. Results: Of 201 patients evaluated, 163 met inclusion criteria: 116 anomalous right CA (71%), 25 anomalous left CA (15%), 17 single CA (10%), and 5 anomalous circumflex CA (3%). Patients presented as an incidental finding (n=80, 49%), with exertional (n=31, 21%) and nonexertional (n=32, 20%) symptoms and following sudden cardiac arrest/shock (n=5, 3%). Eighty-two patients (50.3%) were considered high risk. Predictors of high risk were older age at diagnosis, black race, intramural course, and exertional syncope. Most patients (82%) are allowed unrestrictive sports activities. Forty-seven patients had surgery (11 anomalous left CA and 36 anomalous right CA), 3 (6.4%) remained restricted from sports activities. All patients are alive at a median follow-up of 1.6 (interquartile range, 0.7–2.8) years. Conclusions: In this prospective cohort of patients with anomalous aortic origin of a CA, most have remained free of exercise restrictions. Development of a multidisciplinary team has allowed a consistent approach and may have implications in risk stratification and long-term prognosis.